Serum angiotensin-converting enzyme has low diagnostic value for pediatric sarcoid uveitis

Purpose. - Sarcoid uveitis is rare in the pediatric population. Early diagnosis is challenging and is crucial, due to more severe complications. Diagnosis relies on various criteria, including elevated angiotensin-converting enzyme (ACE) levels. The objective of this study was to evaluate the diagnostic value of serum ACE levels in the diagnosis of pediatric sarcoid uveitis. Methods. - This was an observational retrospective multicenter study of chronic, severe pediatric uveitis between 2013 and 2019 in two French tertiary referral centers. Results. - An ACE assay result was available for 105 patients. Nine patients were diagnosed with sarcoid uveitis. The diagnostic values were as follows: sensitivity = 22.2%, specificity = 87.5%, positive predictive value = 14.3%, negative predictive value = 92.3%, positive likelihood ratio = 1.8, and negative likelihood ratio = 0.9. Conclusion. - The diagnostic performance of ACE in pediatric sarcoid uveitis was found to be poor. NPV exceeded 90% but was based on a significant number of false negatives, indicating a high risk of misdiagnosis. Likelihood ratios confirmed the limited diagnostic value of ACE. Considering age groups and clinical manifestations may enhance precision but requires larger studies. Serum ACE used as a diagnostic marker in pediatric sarcoid uveitis warrants caution and should be interpreted alongside other factors.